What are the symptoms and treatment of acute rheumatic fever?

Symptoms and Treatment of Acute Rheumatic Fever

Acute rheumatic fever (ARF) is characterized by five major manifestations: carditis, arthritis, chorea, erythema marginatum, and subcutaneous nodules, which develop 2-3 weeks following group A streptococcal pharyngitis and require prompt treatment to prevent long-term cardiac complications. 1, 2

Clinical Manifestations

Major Manifestations (Jones Criteria)

1. Carditis (most serious manifestation)

   • Clinical or subclinical (detected by echocardiography)
   • Can lead to valvular heart disease, particularly mitral and aortic valves
   • May present with new murmurs, heart failure, or pericarditis

2. Arthritis

   • Typically migratory and transient
   • Usually affects large joints (knees, ankles, elbows, wrists)
   • Responds rapidly to anti-inflammatory treatment
   • Different from poststreptococcal reactive arthritis (PSRA), which occurs earlier (10 days post-infection) and is more persistent 1

3. Sydenham's Chorea

   • Involuntary, purposeless movements
   • Emotional lability
   • May occur without other manifestations
   • Can appear months after the streptococcal infection

4. Erythema Marginatum

   • Transient, non-pruritic, pink rash with clear centers and rounded edges
   • Primarily on trunk and proximal extremities
   • Appears and disappears rapidly

5. Subcutaneous Nodules

   • Firm, painless nodules over bony prominences
   • Usually associated with carditis

Minor Manifestations

• Fever (≥38.5°C in high-risk populations, ≥38.0°C in low-risk populations)
• Elevated acute phase reactants (ESR ≥60 mm/h or CRP ≥3.0 mg/dL)
• Prolonged PR interval on ECG
• Polyarthralgia
• Evidence of preceding group A streptococcal infection (positive throat culture, rapid antigen test, or elevated streptococcal antibodies) 1

Diagnosis

Diagnosis requires evidence of preceding group A streptococcal infection plus:

• For initial ARF episode: 2 major manifestations OR 1 major and 2 minor manifestations
• For recurrent ARF: Lower threshold - 2 major OR 1 major and 2 minor OR 3 minor manifestations may be sufficient 1

Treatment

1. Eradication of Group A Streptococcal Infection

• First-line: Penicillin (Class I, LOE A)

  • Oral penicillin V: 250 mg 2-3 times daily for 10 days
  • OR Benzathine penicillin G: Single intramuscular injection (1.2 million units for adults; 600,000-900,000 units for children) 1, 3

• For penicillin-allergic patients:

  • Oral erythromycin: 20-40 mg/kg/day (maximum 1 g/day) for 10 days 4
  • OR other macrolides/azalides (clarithromycin, azithromycin) 2

2. Anti-inflammatory Treatment

• Arthritis/Arthralgia:

  • Aspirin: 80-100 mg/kg/day divided into 4 doses (reduce after symptoms improve)
  • OR other NSAIDs for symptom control 5, 6

• Carditis:

  • Corticosteroids for moderate to severe carditis
  • Bed rest during acute phase
  • Heart failure management if present

• Chorea:

  • Valproic acid, carbamazepine, or haloperidol for severe cases
  • Supportive care for mild cases 7

3. Secondary Prophylaxis (Critical)

Secondary prophylaxis must be initiated immediately and continued long-term to prevent recurrences and worsening cardiac damage. 1, 2

• Preferred regimen: Benzathine penicillin G every 3-4 weeks (IM)

  • 1.2 million units for adults
  • 600,000 units for children <27 kg 3

• Alternative oral regimens:

  • Penicillin V: 250 mg twice daily
  • For penicillin-allergic patients: Sulfadiazine (0.5-1 g daily) or erythromycin (250 mg twice daily) 1, 4

Duration of Secondary Prophylaxis

• With carditis and residual heart disease: 10 years or until age 40 (whichever is longer), sometimes lifelong
• With carditis but no residual heart disease: 10 years or until age 21 (whichever is longer)
• Without carditis: 5 years or until age 21 (whichever is longer) 1

Special Considerations

• Family members of ARF patients should be evaluated and treated if positive for group A streptococcus 2
• At least one-third of ARF cases result from asymptomatic streptococcal infections 1
• Recurrences of ARF significantly increase the risk of severe cardiac damage 8
• Patients with poststreptococcal reactive arthritis should be monitored for development of carditis 1

ARF remains a significant cause of acquired heart disease in developing countries, with rheumatic heart disease affecting 25-40% of all cardiovascular disease globally 8, 9. Prompt recognition and appropriate treatment are essential to prevent long-term cardiac sequelae.